[与普通可变免疫缺陷及其他合并症相关的结节性再生性增生]
[Nodular regenerative hyperplasia associated with common variable immunodeficiency and other comorbidities].
作者信息
León Rafael, Sánchez-Martínez Rosario, Palazón José M, Payá Artemio, Ramos José M, Pinargote Héctor
机构信息
Servicio de Medicina Interna, Hospital General Universitario de Alicante, Alicante, España.
Servicio de Medicina Interna, Hospital General Universitario de Alicante, Alicante, España.
出版信息
Med Clin (Barc). 2016 Mar 18;146(6):263-6. doi: 10.1016/j.medcli.2015.11.008. Epub 2015 Dec 23.
BACKGROUND AND OBJECTIVE
Currently, there are not many data on the evolution of nodular regenerative hyperplasia (NRH) associated or not with underlying diseases and in particular that associated with common variable inmunodeficiency (CVID). Twenty cases of NRH are presented, and the differences between the cases associated with CVID and those related to other diseases are analysed.
METHODS
Retrospective and descriptive study over a period of 14 years.
RESULTS
Twelve out of the 20 patients were men; the median age was 51 years. CVID was the main illness associated with NRH. In patients with CVID and NRH, gastrointestinal haemorrhage was more common, all the patients had high gamma glutamyl transferase and alkaline phosphatase and none had altered albumin and bilirubin levels compared to the patients without CVID. On follow-up, 50% of patients with CVID (2/4) had died compared to 33.3% (5/15) without CVID.
CONCLUSIONS
NRH in patients with CVID seems to have more biochemical data of anicteric cholestasis and portal hypertension and could be associated with lower survival.
背景与目的
目前，关于结节性再生性增生（NRH）与潜在疾病相关或不相关的演变情况，尤其是与常见可变免疫缺陷（CVID）相关的情况，数据并不多。本文报告了20例NRH病例，并分析了与CVID相关的病例和与其他疾病相关的病例之间的差异。
方法
进行为期14年的回顾性描述性研究。
结果
20例患者中有12例为男性；中位年龄为51岁。CVID是与NRH相关的主要疾病。在患有CVID和NRH的患者中，胃肠道出血更为常见，所有患者的γ-谷氨酰转移酶和碱性磷酸酶均升高，与无CVID的患者相比，白蛋白和胆红素水平均未改变。随访发现，CVID患者中有50%（2/4）死亡，而无CVID的患者中有33.3%（5/15）死亡。
结论
CVID患者的NRH似乎有更多无黄疸型胆汁淤积和门静脉高压的生化数据，并且可能与较低的生存率相关。
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